Active fault diagnosis (AFD) of parametric faults is considered in connection with closed loop feedback systems. AFD involves auxiliary signals applied on the closed loop system. A fault signature matrix is introduced in connection with AFD and it is shown that if a limited number of faults can occur in the system, a fault separation in the fault signature matrix can be obtained. Then the singl...

Behaviour is a consequence of computation in neural circuits composed of massive synaptic connections among sensory neurons and interneurons. The cyclic AMP response element-binding protein (CREB) responsible for learning and memory is expressed in almost all neurons. Nevertheless, we find that the Caenorhabditis elegans CREB orthologue, CRH-1, is only required in the single bilateral thermosen...

OBJECTIVES We sought to define the prevalence of cardiac involvement in female patients with Anderson-Fabry disease (AFD). BACKGROUND Anderson-Fabry disease is a rare inborn X-linked lysosomal storage disorder. Globotriaosylceramide (Gb(3)), the major substrate of the deficient alpha-galactosidase A enzyme, accumulates progressively in vulnerable cells, including the cardiovascular system. It...

BACKGROUND Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder resulting from the deficiency of trihexosylceramide α-galactosidase (α-Gal A). The diagnosis is often missed or delayed, and specific diagnostic tests (serum α-Gal A activity, genotyping or biopsy) are expensive and not widely available. We evaluated the diagnostic potential of urine microscopy in AFD. METHODS W...

1This research was supported by the Agriculture and Food Research Initiative competitive grant number 2011-68004-30336 form the USDA National Institute of Food and Agriculture. 2Corresponding author: [email protected] ABSTRACT: Little is known of the consequences of divergent selection for residual feed intake (RFI) on intestinal digestion capacity, particularly during immune system stimulati...

The present study examined whether bicultural competence served as a mediator (i.e., an apparent causal mechanism) or a moderator (i.e., a variable that describes for whom or under what situation) for the relationship between Acculturative Family Distancing (AFD) and two psychological outcomes (i.e., depression and psychological wellbeing). A total of 258 Latino college students attending large...

AIMS Anderson-Fabry Disease (AFD), an X-linked disorder of sphingolipid metabolism, is a cause of idiopathic left ventricular hypertrophy but the mechanism of hypertrophy is poorly understood. Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD. METHODS AND RESULTS Eighteen males (mean 43+/-1...

The nematode Caenorhabditis elegans navigates toward a preferred temperature setpoint (Ts) determined by long-term temperature exposure. During thermotaxis, the worm migrates down temperature gradients at temperatures above Ts (negative thermotaxis) and performs isothermal tracking near Ts. Under some conditions, the worm migrates up temperature gradients below Ts (positive thermotaxis). Here, ...

Different interoceptive systems must be integrated to ensure that multiple homeostatic insults evoke appropriate behavioral and physiological responses. Little is known about how this is achieved. Using C. elegans, we dissect cross-modulation between systems that monitor temperature, O₂ and CO₂. CO₂ is less aversive to animals acclimated to 15°C than those grown at 22°C. This difference require...

OBJECTIVES We compared the frequency of a binary endocardial appearance in patients with hypertrophic cardiomyopathy (HCM) and Anderson-Fabry disease (AFD). BACKGROUND A recent study suggested that a binary endocardial appearance is a highly sensitive and specific discriminator of AFD from other causes of hypertrophic cardiomyopathy (HCM). METHODS Fourteen patients with AFD (55.4 +/- 9.9 ye...